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Risk Factors for Death of Patients with Cystic Fibrosis Awaiting Lung Transplantation

Divisions of Pulmonary, Allergy, and Critical Care, and Biostatistics, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania Medical Center; Divisions of Pulmonary Medicine and Oncology, Children's Hospital of Philadelphia; Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania; Division of Pulmonary and Critical Care Medicine, Stanford University Medical Center, Stanford, California; and Division of Respirology, University Health Network and University of Toronto, Toronto, Ontario, Canada

Correspondence and requests for reprints should be addressed to Richard A. Belkin, M.D., Department of Pulmonary and Critical Care Medicine, Sansum-Santa Barbara Medical Foundation Clinic, 215 Pesetas Lane, Santa Barbara, CA 93110. E-mail: rbelkin{at}sansumclinic.org

Rationale: The optimal timing for listing of cystic fibrosis patients for lung transplantation is controversial.

Objectives: We conducted a retrospective cohort study of 343 patients listed for lung transplantation at four academic medical centers to identify risk factors for death while awaiting transplantation.

Methods: Data on possible risk factors were abstracted from medical records.

Measurements: Time to death, patient demographic characteristics, and risk factors for death while awaiting transplantation were assessed. Univariate and multivariate survival analyses were performed using Cox regression.

Results: By univariate analyses, FEV₁ 30% predicted (HR, 3.8; 95% CI, 2.0–7.5), Pa_CO2 50 mm Hg (HR, 1.85; 95% CI, 1.1–3.0), and shorter height (HR, 1.8; 95% CI, 1.1–3.0) were associated with a higher risk of death. Referral from an accredited cystic fibrosis center was associated with a lower risk (HR, 0.53; 95% CI, 0.30–0.92). The final multivariate model included referral from an accredited cystic fibrosis center (HR, 0.5; 95% CI, 0.3–1.0) and listing year after 1996 (HR, 0.4; 95% CI, 0.2–0.7); both were associated with a lower risk of death. FEV₁ 30% predicted (HR, 6.8; 95% CI, 2.4–19.3), Pa_CO2 50 mm Hg (HR, 6.9; 95% CI, 1.5–32.1), and use of a nutritional intervention (HR, 2.3; 95% CI, 1.3–4.1) were associated with increased risk. Patients with FEV₁ > 30% predicted had a higher risk of death only when their Pa_CO2 was 50 mm Hg (HR, 7.0; 95% CI, 1.5–32), while the increased risk of death with FEV₁ 30% was not further influenced by the presence of hypercapnia.

Conclusions: We identified risk factors for waiting list mortality that could impact on transplant listing and allocation guidelines.

Key Words: cystic fibrosis • lung transplantation • survival

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